Author Information

Division of Molecular and Clinical Rheumatology, the Division of Internal Medicine, Department of Medicine, and the Welch Center for Prevention, Epidemiology and Clinical Research, The Johns Hopkins Medical Institutions, Baltimore, Maryland

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Abstract

The course of systemic lupus erythematosus (SLE) is characterized by exacerbations (or flares) and remissions of disease activity. As part of an ongoing prospective cohort study, 3 disease activity indices, the physician's global assessment, the Lupus Activity Index, and the University of Toronto SLE Disease Activity Index, have been recorded, at least quarterly since 1987, on 185 SLE patients. We developed a definition of SLE flare and a description of its clinical epidemiology. Disease flare was defined as a change of ≥1.0 in the physician's global assessment of disease activity (measured on a 0–3 scale) from the previous visit or from a visit within the last 93 days. Of the 185 patients, 98 (53%) had ≥1 flare; the total number of flares was 146. The incidence of flare was 0.65 per patient-year of followup. The median time from the first study visit to a flare was 12 months. Flares were frequently characterized by constitutional symptoms, musculoskeletal involvement, cutaneous involvement, and decreasing levels of C3 and C4. At the time of flare, the mean University of Toronto SLE Disease Activity Index score increased by 3.0 and the mean Lupus Activity Index score (modified to omit the physician's global assessment) increased by 0.26. Overall, 44.8% of the flares prompted a change in treatment. Patients who experienced flares fulfilled more of the SLE criteria at entry and had been followed up for a longer duration after entry into the study, compared with those who did not have flares. By survival analysis, no specific clinical or laboratory variables present at entry were found to predict the time to first flare. These data show that quantification of flare is possible, flare is frequent in patients with SLE of long duration, and the majority of flares involve the so-called “minor” organ systems.